Miscellaneous Metabolic Disorders Age 17 with CC

Cystic fibrosis (CF) is a multisystem genetic disease of the exocrine glands—those glands with ducts such as the mucous, salivary, and sweat-producing glands. CF, originally called CF of the pancreas, is also associated with the glands of the respiratory system and the skin, and it has the potential for multiple organ involvement. The lungs are most frequently affected, but the gastrointestinal (GI) tract (including the small intestine and pancreatic and bile ducts) and eventually the reproductive organs are affected, as well.

CF leads to an increase in viscosity of bronchial and pancreatic secretions, which obstruct the glandular ducts. As thick secretions block the bronchioles and alveoli, the patient develops severe atelectasis (lung collapse) and emphysema. The GI effects of the disease lead to deficiency in the enzymes trypsin, amylase, and lipase. With enzyme deficiency, the conversion and absorption of fats and proteins are altered, and vitamins A, D, E, and K are not properly absorbed. Pancreatic changes include fibrosis, cyst formation, and the development of fatty deposits that lead to pancreatic insufficiency and decreased insulin production. Intelligence and cognitive function are typically not affected.

Complications of CF can be life-threatening. Respiratory complications include lung collapse, pneumothorax, airway collapse, and pneumonia. GI complications include dehydration, malnutrition, gastroesophageal reflux, and rectal prolapse.

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